Encephalitis associated with autoantibody binding to the anti-N-methyl-D-aspartate receptor: immunopathogenesis, mechanisms, and clinical characteristics

Abstract

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis has been increasingly recognized in recent years. This condition may be the most common cause of antibody-mediated encephalitis worldwide. The majority of patients are young at the time of onset, female, and present with an acute-to-subacute onset of behavioral changes followed by seizure, abnormal movement,autonomic dysfunction, and finally hypoventilation with coma if left untreated. The immunopathogenesis of this disease may be due to antibody-mediated internalization of NMDARs from synapses, which results in the dysfunction of particular brain regions(especially the hippocampus and frontostriatal area). Compared to serum, the cerebrospinal fluid permits the more sensitive detection of anti-NMDAR antibody. Ovarian teratoma may be present in up to 40% of patients but is less frequent in children or late-onset disease (> 45 years old). The severity at the time of disease onset and time to appropriate immunotherapy (high-dose steroid plus plasmapheresis or intravenous immunoglobulin) are independent factors that are associated with good outcomes. Copyright © 2016 Neuroimmunology and Neuroinflammation , Published by OAE Publishing Inc.